Bilateral facial capillary malformation associated with eye and brain abnormalities.

OBJECTIVE To establish whether the prognosis of bilateral facial capillary malformation (BFCM) is worse compared with that of unilateral facial port-wine stain. DESIGN Retrospective study. SETTING Paediatric Dermatology Department, Great Ormond Street Hospital for Children NHS Trust, a tertiary referral center for vascular anomalies. PATIENTS A cohort of 350 children who presented with facial CM was seen between January 1, 1994, and June 30, 2004. Twenty-seven children with BFCM were identified. A control group of 27 children with unilateral CM was randomly selected from the total cohort. MAIN OUTCOME MEASURES Demographic, clinical, and radiographic characteristics were recorded and compared between the 2 groups: age at presentation, sex, distribution, extension, extrafacial lesions, glaucoma, ipsilateral leptomeningeal angiomatosis, and epilepsy. The recorded information was collected from the database of the Paediatric Dermatology Department, the hospital records, and the patients' photographs. RESULTS Compared with the 27 children with unilateral facial CM, the 27 with BFCM showed a higher frequency of association with extrafacial lesions (17 [63%] vs 6 [22%]), glaucoma (21 [78%] vs 2 [7%]), and ipsilateral leptomeningeal angiomatosis (14 [52%] vs 2 [7%]). All patients who had BFCM with bilateral and complete involvement of the ophthalmic area had ipsilateral leptomeningeal angiomatosis. CONCLUSION Patients with BFCM must be considered as a group with a worse prognosis compared with patients with unilateral facial CM.